Clinical presentation of Crohn's disease: association between familial disease, smoking, disease phenotype, extraintestinal manifestations and need for surgery

Abstract

Background/Aims: Recent molecular data suggest that genetic factors may underlie the disease heterogeneity observed in Crohn's disease (CD). It was also suggested that familial inflammatory bowel disease (IBD) is a homogenous subgroup, phenotypically different from sporadic disease. Our aim was to determine the clinical presentation in a large CD population. Methodology: 564 CD patients (m/f: 278/286, age: 37.4 (SD 12.7) yrs, duration: 8.4 (7.1) yrs) were included. Disease phenotype was determined according to Vienna classification. Familial disease, extraintestinal manifestations (EIM), need for surgery and smoking habits were also analyzed. Results: Familial IBD was present in 73 (12.9%) patients. Age at onset and presence of EIMs was associated with familial disease. Penetrating (44.6% vs. <10yrs: 29.1%, P<0.0001) and ileocolonic disease (54.4% vs. 42.8%, P=0.03) were more common in patients with a disease duration of ≥10yrs. In a logistic regression model female gender, colonic/ileocolonic location, smoking and familial IBD were independent risk factors for EIMs, while ileal and non-inflammatory disease increased the risk for resections. Smoking was also associated with frequent relapses. Conclusions: Familial IBD was associated with the presence of EIMs, while ileal involvement and noninflammatory behavior independently increased the risk for surgery. Since penetrating and extensive disease was more frequent in patients with longer disease duration our data support a possible change in location and behavior during the course of disease.

Relationship between clinical features of Crohn??s disease and the risk of developing extraintestinal manifestations

“…They were more frequently seen in women than in men (41.1 vs. 26.7%), (odds ratio 1.92, 95% confidence interval: 0.99-3.70; P = 0.05) and in steroid-dependent patients than in steroid responders (61.1 vs. 28.5%), (odds ratio 3.94, 95% confidence interval: 1.83-8.49; P < 0.01). No relationship was found in general between the extraintestinal manifestations of Crohn's disease and smoking habits, previous Crohn's diseaserelated surgery, a family history of Crohn's disease, steroid resistance and CARD15 mutations.…”

Smoking in inflammatory bowel diseases: Good, bad or ugly?

“…However, the harmful effect of smoking on the course of CD is not a universal finding. Studies in patients from Israel and Hungary have not found differences in the need for surgery or for immunosuppressants between smokers and non-smokers [2,44,45] , and patients with only colonic involvement are less sensitive to the harmful effects of smoking [8] . Finally, the development and severity of perineal complications do not seem to be influenced by the smoking status [39] .…”

“…A recent review [36] and previous data have demonstrated that smoking, when measured up to the time point of disease behavior classification, was associated more frequently with complicated disease, penetrating intestinal complications [34,37,38] , and greater likelihood to progress to complicated disease, as defined by development of strictures or fistulae [36] , and a higher relapse rate [2,39] . Of note, previous severity of the disease, as assessed from the therapeutics needs, was found to be similar in young patients who started smoking and in their matched controls [10] .…”

“…Inflammatory bowel disease (IBD) is a multifactorial disease with probable genetic heterogeneity [1,2] . In addition, several environmental (eg, diet, smoking, measles or appendectomy) risk factors may contribute to its pathogenesis.…”

Extraintestinal Manifestations of Inflammatory Bowel Diseases

“…Familial IBD was associated with the presence of EIMs, while ileal involvement and noninflammatory behavior independently increase the risk of surgery (24). Female gender, steroid dependency and colonic involvement are associated with the risk of developing EIMs of CD (19).…”